Italian survey on cardiac surgery for adults with congenital heart disease: which surgery, where and by whom?

OBJECTIVES: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom.METHODS: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units.RESULTS: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons.CONCLUSIONS: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases

Twenty-Year Outcome After The Ross Operation in Neonates, Infants and Children: Results From The Italian Pediatric Ross Registry.

Aim. Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared to adults. In order to define the outcome in the second decade after Ross operation in children, a nation-wide review of 23 years of experience was undertaken.Methods and Results. Three-hundred-and-five children underwent Ross operation in 11 Paediatric units between 1990-2012. Age at surgery was 9.4\ub15.7 years, indication aortic stenosis in 103 patients, regurgitation in 109, mixed lesion in 93. One-hundred-sixteen (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, sub-coronary grafting in 17, Ross/Konno in 73.There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93\ub12% and 85\ub18% and freedom from any reoperation was 76\ub13% and 42\ub110%, at 10 and 20 years. Thirty-four children had autograft 37 reoperations (25 replacement, 12 repair): 3 required transplantation after reoperation. Freedom from autograft reoperation was 86\ub13% and 59\ub110% at 10 and 20 years. Thirty-two children had right-heart redo procedures, only 25 (78%) conduit replacements (20-year freedom from replacement, 77\ub19%). Prior operation (p=0.031), subcoronary implant (p=0.025), concomitant surgical procedure (p=0.004) were risk factors for left-heart reoperation, while infant age (p=0.015), for right-heart. Majority (87%) of late survivors were in NYHA class I, 68% free from medication and 6 women had pregnancies.Conclusion. Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity at 20 years. Contrary to expectation, autograft reoperation is more common than homograft. Further patient selection and modification of operative techniques may decrease valve-related events

Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry.

Objective: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23\u2005years of experience was undertaken.Methods: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4\ub15.7\u2005years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross\u2013Konno in 73.Results: There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7\u2005years). Survival was 93\ub12% and 89\ub13% and freedom from any reoperation was 76\ub13% and 67\ub16% at 10 and 15\u2005years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86\ub13% and 75\ub16% at 10 and 15\u2005years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89\ub14%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies

Factors associated with perioperative mortality in children and adolescents operated for tetralogy of Fallot: A sub-Saharan experience

BACKGROUND: Patients with tetralogy of Fallot are now surviving to adulthood with timely surgical intervention. However, many patients in low-income countries have no access to surgical intervention. This paper reports the surgical access and perioperative mortality in a sub-Saharan center that was mainly dependent on visiting teams. METHODS: We reviewed records of patients operated from January 2009 to December 2014. We examined perioperative outcomes, primarily focusing on factors associated with perioperative mortality. RESULTS: During this period, 62 patients underwent surgery. Fifty-seven (91.9%) underwent primary repair, while 5 (6.5%) underwent palliative shunt surgery. Of the five patients with shunt surgery, four ultimately underwent total repair. Eight (12.9%) patients died during the perioperative period. Factors associated with perioperative mortality include repeated preoperative phlebotomy procedures (P \u3c .001), repeated runs and long cardiopulmonary bypass time (P \u3c .001), and aortic cross-clamp time (P \u3c .001), narrow pulmonary artery (PA) valve annulus diameter (P = .022), narrow distal main PA diameter (P = .039), narrow left branch PA diameter (P = .049), and narrow right PA diameter (P = .039). Of these factors, cardiopulmonary bypass time/aortic cross-clamp time and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality. CONCLUSION: In this series of consecutive patients operated by a variety of humanitarian surgical teams, cardiopulmonary bypass time/aortic cross-clamp time, and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality risk. As some of these factors are modifiable, we suggest that they should be considered during patient selection and at the time of surgical intervention